Journal of Otology & RhinologyISSN: 2324-8785

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Case Report, J Otol Rhinol Vol: 5 Issue: 5

Spontaneous Cochlear Hemorrhage in an otherwise Healthy Patient: A Case Report

Molly L Naunheim* and Marika D Russell
Department of Otolaryngology-Head and Neck Surgery, University of California- San Francisco Medical Center, San Francisco, CA, USA
Corresponding author : Molly Naunheim, MD
Department of Otolaryngology- Head and Neck Surgery, University of California-San Francisco Medical Center, 2380 Sutter Street, 3rd Floor; San Francisco, CA 94115, USA
Tel:
415-476-4952
Fax:
415-885-7546
E-mail: [email protected]
Received: September 26, 2016 Accepted: October 06, 2016 Published: October 13, 2016
Citation: Naunheim ML, Russell MD (2016) Spontaneous Cochlear Hemorrhage in an otherwise Healthy Patient: A Case Report. J Otol Rhinol 5:5. doi:10.4172/2324-8785.1000293

Abstract

Cochlear hemorrhage is a rare event that results in permanent sensorineural hearing loss. Most often, this injury is secondary to a traumatic event, though blood dyscrasias have also been implicated. We present a case of a 34 year-old otherwise healthy female who suffered what appears to be a spontaneous cochlear hemorrhage, resulting in significant hearing loss. No trauma preceded the event. She had no known underlying coagulopathies or hypercoagulation disorder. Laboratory studies since the event have been unrevealing. This case demonstrates the possibility of this unfortunate event occurring spontaneously.

Keywords: Cochlear hemorrhage; Sensorineural hearing loss

Keywords

Cochlear hemorrhage; Sensorineural hearing loss

Introduction

Cochlear hemorrhage is an unusual but well-described etiology of sudden sensorineural hearing loss. In previous case reports, several identifiable causes have been implicated; these etiologies typically are associated with blood dyscrasias or trauma. More specifically, causes include sickle-cell disease [1], leukemia [2], trauma (both accidental and iatrogenic) [3-5], and anticoagulation therapy [6]. To our knowledge, no cases of spontaneous cochlear hemorrhage have yet been reported.

Case Report

A 34 year-old otherwise healthy female presented to the emergency department (ED) due to 10 days of right ear pain and decreased hearing; she also reported 5 days of constant vertigo with associated nausea and vomiting. Exam by the ED was notable for a normal ear exam, no nystagmus, and no focal neurological deficits, but she was noted to have a slightly altered heel-toe walk. Computed tomography (CT) of her temporal bone was unremarkable. She was discharged with a referral to otolaryngology and an audiogram appointment. No treatments were prescribed.
A few weeks later, she was seen in the otolaryngology clinic, where a more detailed history was elicited. The patient expressed that six weeks prior, she acutely lost hearing in her right ear. This event occurred during the daytime, “all of a sudden”. No trauma, noise exposure, or upper respiratory symptoms preceded this. Ringing right-sided tinnitus developed at the same time. Nausea and vomiting developed a few days after; these symptoms were constant at first. The patient stated that she now experienced discrete episodes of nausea and vomiting for about twenty minutes a day but that these episodes were becoming less severe and less frequent with time.
The patient was otherwise healthy. Specifically, she had no known underlying autoimmune disease, bleeding or coagulation disorders, familial history of hearing loss, or personal history of miscarriage, which can be a sign of lupus anticoagulant disorder (in fact, she had three healthy children). She was on no anti-platelet or anti-coagulant medication.
Audiogram at that time showed profound sensorineural hearing loss in right ear and normal hearing up to 4,000 hertz on the left (Figure 1). A magnetic resonance image (MRI) with and without gadolinium contrast was ordered to rule out a retrocochlear process. Given the number of weeks since onset, steroid therapy was not offered to this patient.
Figure 1:Patient’s pure-tone audiogram demonstrating a profound rightsided sensorineural hearing loss. Frequency in hertz is shown across the x-axis. Intensity in decibels is depicted along the y-axis. “X” represents the left ear. “Δ” represents the right ear.
The MRI was notable for intrinsic hyper intensity on T1-weighted imaging in the right cochlea (Figure 2), concerning for blood products in the basal turn of the cochlea. This hyper intensity was also clearly demonstrated on the T1-weighted post-gadolinium image in a coronal plane, demonstrating the classic spiral of the cochlea (Figure 3). Of note, this MRI was obtained approximately two months after the initial insult.
Figure 2: Axial non-contrast MRI T1-weighted pre-gadolinium. Intrinsic hyperintensity can be seen in the right cochlea. An enlarged image of this finding is shown in the lower right corner.
Figure 3: Coronal T1-weighted post-gadolinium with fat saturation. The spiral of the cochlea can be seen distinctly on this image. An enlarged image of this finding is shown in the lower right corner.
The patient returned to otolaryngology clinic to discuss these results one month later. Her nausea and vomiting had abated. Further testing was ordered in order to rule out hematologic pathology (complete blood count with a differential, blood smear, hemoglobinopathy evaluation, coagulation panel, and Russell viper venom). All of these tests were unremarkable.
Her repeat audiograms several months out from presentation to the ED showed no improvement in her hearing. She was fitted with a hearing aid, and is currently undergoing evaluation for cochlear implantation.

Discussion

Cochlear (as well as intralabyrinthine) hemorrhage is a rare and unfortunate cause of sensorineural hearing loss, as hearing recovery is generally thought to be unlikely. Vestibular recovery following intralabyrinthine hemorrhage has been described in one report [7]; in general, however, compensation and acclimation to the deficit allows for meaningful function of the vestibular system.
Underlying causes of intralabyrinthine hemorrhage are varied, and yet, to our knowledge, these etiologies have always been identifiable; this is the first reported case of what appears to be a spontaneous cochlear hemorrhage.
Previous case reports describe this injury following traumatic events whether due to blunt force trauma [5], barotrauma [4] or iatrogenic causes [3], similar symptoms are observed – acute permanent hearing loss, and often, vestibular symptoms as well.
Blood dyscrasias account for another previously described etiology of intralabyrinthine hemorrhage. Leukemia [2], sickle-cell [1] and lupus [8] have all been implicated in the pathogenesis of this event. Intrinsic vasculidities [9] have also been noted as potential causes of intralabyrinthine hemorrhage.
After completing a thorough workup, no cause has yet been identified in our patient. It is certainly possible that she may have had an occult vacuity that is not readily diagnosed on laboratory testing, but she has had no other sequelae from this. At this point in time, her hemorrhage is understood as spontaneous.
Though the MRI demonstrated evidence of only cochlear hemorrhage, our patient likely suffered a labyrinthine hemorrhage given the degree of her vestibular symptoms. This acute picture of labyrinthitis may also explain her acute otalgia upon presentation. Blood products in the vestibule may have reabsorbed by the time the MRI was obtained.
Another notable finding is the absence of any findings on CT. One is classically taught that acute hemorrhage should have intrinsic hyper density upon CT. Whether due to volume averaging or due to a true lack of signal, there was no abnormality noted on the CT temporal bone. This may indicate that the patient had a more slow bleeding process, as MRI is more sensitive for micro bleeds and chronic hemorrhage [10].
Acute unilateral hearing loss typically prompts an urgent audiogram and subsequent treatment with high-dose steroids if acute sensorineural hearing loss is confirmed. It is not clear that administration of steroids in this case would have changed the outcome. Vascular causes of sudden sensorineural hearing loss (SSNHL) have long been posited, though the causes are often thought to be ischemic, not hemorrhagic. Hemorrhagic transformation cannot be ruled out, however, as blood products can reabsorb by the time an MRI is obtained. One could argue that identifying blood products in the labyrinth upon a workup of SSNHL might assist with counseling patients regarding the expected benefit of steroid therapy.
Though the cochlear hemorrhage in this case was likely spontaneous, one should remain alert to the possibility of underlying blood dyscrasias in patients who develop acute hearing loss and symptoms of labryinthitis. An MRI can be useful in determining the presence of blood products in the cochlea, and further appropriate work-up should follow.
In conclusion, cochlear hemorrhage is a rare event that results in permanent hearing loss. Most often, this injury follows a traumatic event, though blood dyscrasias have also been implicated. We present a case demonstrating the possibility of this unfortunate event occurring spontaneously.

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